Contributions of BMPR2 Mutations and Extrinsic Factors to Cellular Phenotypes of Pulmonary Arterial Hypertension Revealed by Induced Pluripotent Stem Cell Modeling

Abstract excerpt

Reduced BMPR2 (bone morphogenetic protein receptor 2) signaling is central to the pathobiology of pulmonary arterial hypertension (PAH).However, the reduced penetrance of BMPR2 mutations in families suggests that other factors are required to establish disease (1).To date, it has proved difficult to elucidate these factors because of a lack of appropriate models.Sa and colleagues (2) developed an induced...

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